P149 The intracellular chloride channel 4 (CLIC4) plays an important role in systemic sclerosis fibroblast activation

Abstract Background/Aims The intracellular chloride ion channel CLIC4 mediates the activation of cancer associated fibroblasts. Interestingly, systemic sclerosis (SSc) fibroblasts display a number similar properties to Tissue fibrosis in SSc is driven by active (myofibroblasts). Therefore this study we investigated role fibroblast activation. Methods Dermal were obtained from full thickness skin biopsies patients (early-diffuse). RNA and protein collected transcript levels assessed qPCR western blot. patient treated with inhibitors NPPB IAA-94. Results was found be expressed at significantly higher compared healthy controls, both (3.7 fold) (1.7 levels. Inhibition TGF-β signalling pathway led reduced expression fibroblasts, confirming as main driver expression. Finally, treatment small molecule that target myofibroblast markers collagen type 1 alpha-smooth muscle actin, suggesting direct for fibrosis. Conclusion We have identified novel activation, which further strengthen similarities between Furthermore highlights therapeutic intervention. Disclosure C. Wasson: None. R. Ross: Morton: J. Mankouri: F. Del Galdo: